Optic neuritis & optic atrophy

Optic neuritis & optic atrophy

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  Moderator: Dr. (prof.)P. RAWAT Ma’m Presenter: Dr. SAMIKSHA
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  OPTIC NEURITIS
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  DEFINITION An inflammation ofthe optic nerve is known as OPTIC NEURITIS
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  AETIOLOGY
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  CLASSIFICATION A) According toOPHTHALMOSCOPIC APPEARANCE  Retrobulbar neuritis  Papillitis  Neuroretinitis
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  B) According tothe etiology  Demyelinating (MOST COMMON)  Parainfectious  Infectious  Non infectious
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  A.1 RETROBULBAR NEURITIS the optic disc appears normal, initially, because the optic nerve head is not involved  It is the most common type in adults and is frequently associated with multiple sclerosis (MS).
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  Demyelinating conditions that mayinvolve the visual system include the following:  Isolated optic neuritis with no clinical evidence of generalized demyelination although in a high proportion of cases this subsequently develops
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   Devic disease(neuromyelitis optica), characterized by bilateral optic neuritis and the subsequent development of transverse myelitis (demyelination of the spinal cord) within days or weeks.
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   Schilder disease,a progressive generalized disease with an onset prior to the age of 10 years and death within 1–2 years. Bilateral optic neuritis without subsequent improvement may occur.
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   idiopathic demyelinatingdisease involving central nervous system white matter.  It is more common in women than men.  Presentation is typically in the third–fourth decades, generally with relapsing/remitting demyelination that may switch later to an unremitting pattern, and less commonly with progressive disease from the outset.
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  Systemic features mayinclude: o Spinal cord o Brainstem o Cerebral o Psychological o Lhermitte sign (electrical sensation on neck flexion)
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  Ophthalmic features:  Common.Optic neuritis (usually retrobulbar)  Uncommon. Skew deviation, ocular motor nerve palsies, hemianopia.  Rare. Intermediate uveitis and retinal periphlebitis  Uhthoff phenomenon (sudden worsening of vision or other symptoms on exercise or increase in body temperature)  Pulfrich phenomenon (altered perception of moving objects)
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  Investigation:. ○ Lumbar punctureshows oligoclonal bands on protein electrophoresis of cerebrospinal fluid in 90–95% cases ○ MRI almost always shows characteristic white matter lesions ○ VEPs are abnormal (conduction delay and reduction in amplitude) in up to 100% of patients with clinically definite MS
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  Multiple sclerosis. T1-weightedaxial MR image showing characteristic periventricular plaques
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  Oligoclonal bands onprotein electrophoresis
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  Clinical features of demyelinatingoptic neuritis SYMPTOMS ○ Subacute monocular visual impairment. ○ age range 20–50 years ○ tiny white or coloured flashes or sparkles (phosphenes). ○ Discomfort or pain in or around the eye, exacerbated by ocular movement;it may precede or accompany the visual loss and usually lasts a few days. ○ Frontal headache and tenderness of the globe
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  SIGNS ○ Visual acuity(VA) is usually 6/18–6/60, but may rarely be worse. ○ impaired colour vision and a relative afferent pupillary defect. ○ The optic disc is normal in the majority of cases (retrobulbar neuritis), the remainder show papillitis ○ Temporal disc pallor may be seen in the fellow eye, indicative of previous optic neuritis
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  VISUAL FIELD DEFECTS ○Diffuse depression of sensitivity in the entire central 30° is the most common. ○ Altitudinal/arcuate defects and focal central/centrocaecal scotomas are also frequent.
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  COURSE: Vision worsens overseveral days to 3 weeks and then begins to improve. Initial recovery is fairly rapid and then slower over 6–12 months.
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  PROGNOSIS: ○ More than90% of patients recover visual acuity to 6/9 or better. ○ colour vision, may remain abnormal. ○ A mild relative afferent pupillary defect may persist. ○ Temporal optic disc pallor or more marked optic atrophy may ensue. ○ About 10% develop chronic optic neuritis with slowly progressive or stepwise visual loss.
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  TREATMENT FOLLOWING DEMYELINATING OPTICNEURITIS: • Indications for steroid treatment. When visual acuity within the first week of onset is worse than 6/12, treatment may speed up recovery by 2–3 weeks and may delay the onset of clinical MS. • Steroid regimen. Intravenous methylprednisolone sodium succinate 1 g daily for 3 days, followed by oral prednisolone (1 mg/kg daily) for 11 days, subsequently tapered over 3 days. Oral prednisolone may increase the risk of recurrence of optic neuritis if used without prior intravenous steroid.
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  • Immunomodulatory treatment(IMT) Reduces the risk of progression to clinical MS in some patients, but the risk versus benefit ratio has not yet been fully defined with the options available, which include interferon beta, teriflunomide and glatiramer
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  A.2 PAPILLITIS  Itaffects the optic nerve head (hyperemia & oedema of optic disc) along with peripapillary flame shaped haemorrhages  The disc is at first hyperaemic; later the margins become blurred, oedema ensue which spread onto the retina, the retinal veins become tortuous and extensively distorted, exudates may accumulate upon the disc and there are fine vitreous opacities
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  CLINICAL FEATURES:  profoundvisual loss  disturbance of colour vision  decrease in contrast sensitivity  diminished stereoacuity  abnormal visual fields  Pupillary reactions demonstrate a prominent relative afferent pupillary defect
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  INFLAMMATION MILD INFECTION RESOLVED NORMAL OPTIC DISC SEVERE OR PROLONGED DESTRUCTION OFNERVE FIBRES POST NEURITIC ATROPHY
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  POST NEURITIC ATROPHYshowing blurred disc margins, dirty grey colour floor filled in with organized tissue which extends onto the constricted arteries as perivascular sheaths
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  A.3 NEURO RETINITIS Inflammation of the optic disc with adjacent retinal inflammation  papillitis with retinal exudates which are in the retinal nerve fibre layer are usually radially oriented forming a macular fan or star.  It is a combination of optic neuritis and signs of retinal, usually macular, inflammation.  60% of cases are due to CAT SCRATCH FEVER.  25% of cases are idiopathic(Leber idiopathic stellate neuroretinitis).  Other causes include syphilis, Lyme disease, mumps and leptospirosis
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  SYMPTOMS Painless unilateral visualimpairment, gradually worsening over a week. SIGNS ○ Impaired visual acuity ○ Signs of optic nerve dysfunction are usually mild or absent, as visual loss is largely due to macular involvement. ○ Papillitis associated with peripapillary and macular oedema ○ A macular star appears as disc swelling settles; the macular star resolves with a return to normal or near- normal visual acuity over 6–12 months. ○ Venous engorgement and splinter haemorrhages may be present in severe case. ○ Fellow eye involvement occasionally develops
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  Progression of neuroretinitis. (A)Severe papillitis; (B) later stage showing macular star
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  • Optical coherencetomography (OCT) Shows sub- and intraretinal fluid to a variable extent. • Fluorescein angiography (FA) shows diffuse leakage from superficial disc vessels. • Blood tests may include serology for Bartonella and other causes according to clinical suspicion
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  Treatment  This isspecific to the cause, and often consists of antibiotics.  Recurrent idiopathic cases may require treatment with steroids
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  B.1 DEMYELINATING OPTICNEURITIS  Most common type  Frequently associated with MULTIPLE SCLEROSIS (discussed earlier)
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  B.2 PARAINFECTIOUS OPTICNEURITIS Optic neuritis may be associated with 1.viral infections such as measles, mumps, chickenpox, rubella, whooping cough and glandular Fever 2. following immunization *Children>Adults
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  PRESENTATION  usually 1–3weeks after a viral infection acute severe visual loss generally involving both eyes  Bilateral papillitis is the rule  occasionally there may be a neuroretinitis or the discs may be normal. PROGNOSIS  spontaneous visual recovery is very good  treatment is not required in the majority of patients  when visual loss is severe and bilateral or involves an only seeing eye, intravenous steroids should be considered, antiviral cover where appropriate.
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  MEASLES  Subacute sclerosingpanencephalitis (SSPE) is a late complication of measles infection, manifesting with chronic progressive neurodegenerative & usually fatal disease of childhood  Posterior uveitis is common, and may be the presenting feature
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  Retinal involvement insubacute sclerosing panencephalitis
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  RUBELLA  ocular featuresof congenital rubella include cataract, anterior uveitis, ‘salt and pepper’ pigmentary retinopathy, glaucoma and microphthalmos
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  Rubella retinopathy
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  B.3 INFECTIOUS OPTICNEURITIS a) SINUS RELATED OPTIC NEURITIS  is uncommon  characterized by recurrent attacks of unilateral visual loss  associated with severe headache and spheno- ethmoidal Sinusitis  Possible mechanisms include direct spread of infection, occlusive vasculitis and mucocoele  Treatment : systemic antibiotics surgical drainage
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  b) CAT SCRATCHFEVER  caused by Bartonella henselae  transmitted by the scratch (or bite) of an apparently healthy cat  One or more red papules at the site of inoculation are followed by fever and regional lymphadenopathy
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  Ocular features  NEURORETINITISis the most common manifestation  disc oedema with macular exudate in a star conformation  intermediate uveitis  focal retinochoroiditis  Vasculitis  conjunctivitis with a 2–4 mm conjunctival granuloma (Parinaud oculoglandular Syndrome)
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  Cat-scratch disease –ulcerated papule on the cheek caused by a cat scratch 2 weeks previously, with enlargement of submandibular lymph nodes
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  Treatment  Oral antibiotics,e.g. co-trimoxazole, azithromycin, rifampicin or ciprofloxacin (avoided in children)  Steroids  Treatment is not usually given for mild systemic symptoms alone.
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  c) SYPHILIS  causedby Treponema pallidum  It may cause acute papillitis or neuroretinitis during the primary or secondary stages
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  Ocular features  Anterioruveitis  Chorioretinitis is often multifocal bilateral and associated with vitritis  Acute syphilitic posterior placoid chorioretinopathy (ASPPC) is characterized by large pale-yellowish subretinal lesions in the posterior pole  Retinitis has a ‘ground glass’ appearance  Optic neuritis and neuroretinitis  Other features include conjunctivitis, episcleritis and scleritis, intermediate uveitis, glaucoma, cataract and miscellaneous neuro-ophthalmic features related to CNS involvement including Argyll Robertson pupils
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  ROSEOLAE
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  old multifocal chorioretinitis
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  acute posterior placoidchorioretinitis
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  NEURORETINITIS
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  d) LYME DISEASE Caused by Borrelia burgdorferi  transmitted by a tick bite  It may cause neuroretinitis and occasionally acute retrobulbar neuritis, which may be associated with other neurological manifestations and can mimic MS.
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  Fundus photograph ofpatient having Lyme disease
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  e) CRYPTOCOCCAL MENINGITIS •Cryptococcalmeningitis in patients with acquired immunodeficiency syndrome (AIDS) may be associated with acute optic neuritis, which may be bilateral
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  Severe optic nervehead oedema in a patient with CRYPTOCOCCAL MENINGITIS
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  Optic atrophy &severe visual loss after treatment of cryptococcal meningitis
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  F) VARICELLA ZOSTERVIRUS  may cause papillitis by spread from contiguous retinitis or associated with herpes zoster ophthalmicus.  Primary optic neuritis is uncommon but may occur in immunocompromised patients
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  A) SARCOIDOSIS  Theoptic nerve head may exhibit a lumpy appearance suggestive of granulomatous infiltration and there may be associated vitritis  The response to steroid therapy is often rapid, though vision may decline if treatment is tapered or stopped prematurely, some patients require long-term low-dose therapy.  Methotrexate may also be used as an adjunct to steroids or as monotherapy in steroid-intolerant patients. B.4 NON INFECTIOUS OPTIC NEURITIS
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  Sarcoid granuloma ofthe optic nerve head with overlying vitreous haze
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  B) AUTOIMMUNE  Autoimmuneoptic nerve involvement may take the form of retrobulbar neuritis or anterior ischaemic optic neuropathy  Some patients may also experience slowly progressive visual loss suggestive of compression.  Treatment is with systemic steroids and other immunosuppressants.
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  PARASITIC INFESTATIONS OF THEOPTIC NERVE  Cysticercus cellulosae within the optic nerve is rare.  there is profound visual loss  the condition may mimic optic neuritis, papillitis, neuroretinitis or unilateral severe disc oedema.  the diagnosis is often delayed or missed as it is often mistaken for an optic nerve tumour on neuroimaging.
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  Fundus photograph showingsevere disc oedema with inflammation
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  CT scan ofthe same patient shows a cystic lesion within the optic nerve with an internal, hyperdense lesion suggestive of a scolex.
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   The cysthas a highly reflective pinhead lesion within it representing the scolex which may be detected by ultrasonography or careful examination of CT or MRI scans performed with 1 mm sections
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  TREATMENT  high dosesof STEROIDS to reduce inflammation as the toxins released by the dying parasite are believed to be responsible for the visual loss.  Medical treatment: ORAL ALBENDAZOLE  surgical removal of the cyst (poor results)
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  DIFFERENTIAL DIAGNOSIS  Ischemicoptic neuropathy  Papilloedema  Leber hereditary optic neuropathy  Toxic optic neuropathy  metabolic optic neuropathy  compressive space occupying lesion in the orbit  Compressive space occupying lesion intracranially in the chiasmal region
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  CLINICAL WORKUP  SYMPTOMS(discussed earlier)  careful HISTORY TAKING  AGE of the patient  RAPIDITY OF ONSET  occurrence of any PREVIOUS EPISODES  presence of PAIN ON EYE MOVEMENTS
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  COMPLETE OPHTHALMIC EXAMINATION recording the VISUAL ACUITY  COLOUR VISION  assessing PUPILLARY REACTIONS  RETINAL EXAMINATION to assess the optic nerve  EVALUATION OF VITREOUS for cells.  VISUAL FIELD testing  VEPs recorded for both eyes, as asymptomatic fellow eye abnormalities are common and can be detected with these techniques
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  ADDITIONAL TESTS (performedfor ‘atypical’ optic neuritis)  complete blood count  estimation of rapid plasma reagin  CRP  ESR  fluorescent treponemal antibody absorption (FTA-ABS) test  antinuclear antibody (ANA) test.
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   For thefirst episode and in every atypical case, MRI of the brain and orbits with gadolinium enhancement is recommended to predict the likelihood of MS and ruling out a space-occupying lesion.  Patients with demyelination of the central nervous system on MRI or an abnormal neurological examination should be referred to a neurologist for evaluation and management of possible multiple sclerosis.
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  TREATMENT (based onONTT) 1. profound visual loss + no previous history of optic neuritis or multiple sclerosis seen early + MRI shows at least one area of demyelination, I.V METHYLPREDNISOLONE (250 mg intravenously slowly over 30–60 minutes repeated 6 hourly for 3 days, followed by oral prednisolone 1 mg/kg/day for 11 days) Prednisolone is rapidly tapered off over the next 3 days.
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  If the MRIis normal, multiple sclerosis is unlikely but cannot be entirely ruled out. Pulsed intravenous steroid treatment may still be used to shorten the period of visual impairment, particularly in severe and bilaterally affected cases. Oral prednisolone, in conventional doses of 1 mg/kg/day, should never be used alone as the recurrence rate is remarkably high
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  2. If apatient has already been diagnosed to have multiple sclerosis or has suffered from prior episodes of optic neuritis,Observation is the rule, unless faster visual recovery is specifically required.
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  OPTIC ATROPHY
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  DEFINITION  Optic atrophyrefers to the late stage changes that take place in the optic nerve resulting from axonal degeneration in the pathway between the retina and the lateral geniculate body, manifesting with disturbance in visual function and in the appearance of the optic nerve head.
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   It canbe classified in several ways, including by whether axonal death is initiated in the retina (ANTEGRADE) or more centrally (RETROGRADE) & by cause  A brain tumour will produce primary atrophy if it presses upon the chiasma or optic nerve, and a secondary optic atrophy if it causes papilloedema due to increased intracranial pressure.
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  1) PRIMARY OPTICATROPHY  occurs without antecedent swelling of the optic nerve head.  It may be caused by lesions affecting the visual pathways at any point from the retrolaminar portion of the optic nerve to the lateral geniculate body.  Lesions anterior to the optic chiasm result in unilateral optic atrophy, whereas those involving the chiasm and optic tract will cause bilateral changes.
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  • Signs  Flatwhite disc with clearly delineated margins  Reduction in the number of small blood vessels on the disc surface.  Attenuation of peripapillary blood vessels and thinning of the retinal nerve fibre layer (RNFL).  The atrophy may be diffuse or sectoral depending on the cause and level of the lesion.  Temporal pallor of the optic nerve head may indicate atrophy of fibres of the papillomacular bundle, classically seen following demyelinating optic neuritis.  Band atrophy is caused by involvement of the fibres Entering the optic disc nasally and temporally (occurs in lesions of the optic chiasm or tract and gives nasal as well as temporal pallor)
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  Primary optic atrophydue to compression
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  • Important causes Optic neuritis.  Compression by tumours and aneurysms.  Hereditary optic neuropathies.  Toxic and nutritional optic neuropathies may give temporal pallor  Trauma.
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  primary optic atrophydue to nutritional neuropathy – note predominantly temporal pallor;
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  Optic atrophy showingdemyelination of the fibres from the papillomacular bundle.
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  2) SECONDARY OPTIC ATROPHY Secondary optic atrophy is preceded by long- standing swelling of the optic nerve head.
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  • SIGNS varyaccording to the cause and its course.  Slightly or moderately raised white or greyish disc with poorly delineated margins due to gliosis  Obscuration of the lamina cribrosa.  Reduction in the number of small blood vessels on the disc surface.  Peripapillary circumferential retinochoroidal folds, especially temporal to the disc (Paton lines)  sheathing of arterioles and venous tortuosity may be present.
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  secondary optic atrophydue to chronic papilloedema – note prominent Paton lines
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  CAUSES  chronic papilloedema anterior ischaemic optic neuropathy  papillitis.  Intraocular inflammatory causes of marked disc swelling
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  3) CONSECUTIVE OPTICATROPHY  caused by disease of the inner retina or its blood supply.  The cause is usually obvious on fundus examination, e.g. extensive retinal photocoagulation, retinitis pigmentosa or prior central retinal artery occlusion.  The disc appears waxy.
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  consecutive optic atrophydue to vasculitis
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  4) GLAUCOMATOUS OPTICATROPHY A) SUBTYPES OF GLAUCOMATOUS DAMAGE i) Focal ischaemic discs characterized by  localized superior and/or inferior notching  may be associated with localized field defects
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  Focal ischaemic –inferior notch and disc haemorrhage
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  ii) Sclerotic discsare characterized by:  a shallow saucerized cup and a gently sloping NRR  Variable peripapillary atrophy  peripheral visual field loss  associated with systemic vascular disease.
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  sclerotic
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   iii) Myopicdisc with glaucoma  refers to a tilted (obliquely inserted) shallow disc  temporal crescent  parapapillary atrophy
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  myopic